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一名慢性淋巴细胞白血病和单克隆丙种球蛋白病患者的弥漫性扁平正常血脂性黄瘤病

Diffuse plane normolipaemic xanthomatosis in a patient with chronic lymphatic leukaemia and monoclonal gammopathy.

作者信息

Stockman Annelies, Delanghe Joris, Geerts Marie-Louise, Naeyaert Jean Marie

机构信息

Department of Dermatology, University of Ghent, Belgium.

出版信息

Dermatology. 2002;204(4):351-4. doi: 10.1159/000063384.

Abstract

Diffuse plane normolipaemic xanthomatosis is a well-defined uncommon condition characterized by yellow-orange plaques involving the eyelids, neck, upper trunk, buttocks and flexural folds. Over half of the reported cases are associated with lymphoproliferative disorders. The condition may arise as a result of perivascular deposition of lipoprotein-immunoglobulin complexes. We present a female patient with clear clinical features of diffuse plane xanthomatosis associated with chronic lymphatic leukaemia and monoclonal gammopathy. In this patient, we found evidence of complement consumption supporting the hypothesis that complexes of lipoprotein and paraprotein are formed. Lipid electrophoresis of fasting serum revealed the presence of a faint precipitate at the application slot, also indicative of the presence of protein-lipid complexes.

摘要

弥漫性扁平正常血脂性黄瘤病是一种明确的罕见病症,其特征为眼睑、颈部、上躯干、臀部及屈侧皱襞出现黄橙色斑块。超过半数的报告病例与淋巴增殖性疾病相关。该病症可能是脂蛋白 - 免疫球蛋白复合物血管周围沉积的结果。我们报告一名具有弥漫性扁平黄瘤病明确临床特征的女性患者,其与慢性淋巴细胞白血病和单克隆丙种球蛋白病相关。在该患者中,我们发现了补体消耗的证据,支持脂蛋白和副蛋白复合物形成的假说。空腹血清的脂质电泳显示加样槽处有微弱沉淀,这也表明存在蛋白质 - 脂质复合物。

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